• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1358-1362
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• 2006

Niemann-Pick disease is a group of autosomal recessive disorders associated with hepatosplenomegaly, variable neurologic deficits, and the storage of sphingomyelin and other lipids. Seven cases have been reported in Korea. We report an additional case presenting with hypotonia, early neurodevelopmental delay, hepatosplenomegaly and death by persistent pneumonia and asphyxia at the age of 23 months. MRI of brain and fundoscopic findings of our case at 4 months of age were normal. However, abnormal intensity of the thalamus and atrophy of the right temporal lobe on the MRI and macular cherry red spots were noticed at the age of 17 months. A bone marrow biopsy showed large foamy cells, while hexosaminidase A and B levels were normal. Although biochemical or molecular workup was not done, these findings led to the diagnosis of infantile onset Niemann-Pick disease, probably type A. A brief review of the related literatures was made.

• The Korean Journal of Cytopathology
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• v.7 no.1
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• pp.97-102
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• 1996

Fine needle aspiration cytology of a pulmonary mass was performed on a 51-year-old man who had a left testicular mass. Cytologic features were composed of a homogeneous population of malignant cells associated with a background of foamy and lacelike material. The cellular features were characterized by monomorphous cell proliferation of relatively regular large cells, generally isolated or grouped. Occasionally, fine blanching stroma with large tumor cells and scanty lymphocytes were noted. The tumor cells had a round, regular nucleus, prominent round nucleoli, and a thin rim of cytoplasm containing large vacuoles or lacunae filled with glycogen. The fine needle aspiration cytologic diagnosis was highly consistent with metastatic seminoma from testis and less likely primary or other metastatic carcinoma. The diagnosis of resected testicular mass was classic seminoma. Despite the fact that cytopathologists were not familial with diagnosis of seminoma due to clinician's lack of interest in fine needle aspiration cytology of germ cell tumors including seminoma, it appears that a diagnosis of this tumor should not be problematic in cytologic material if specific histologic criteria are applied.

• Tuberculosis and Respiratory Diseases
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• v.41 no.1
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• pp.51-57
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• 1994

Amiodarone is a potent antiarrythmic agent used in the treatment of refractory tachyarrythmias and premature ventricular contractions. Amiodarone may be responsible for the frequent and various side effects including corneal deposits, abnormal liver function test, hyperthyriodism or hypothyroidism, bluish discoloration of the skin, neuropathies, and the others. However, pulmonary toxicity is most serious adverse reaction limiting the clinical efficacy. Rescently, we experienced a case of pulmonary toxicity induced by low dose 10-month amiodarone treatment for atrial fibrilation with rapid ventricular response. Lung biopsy reveals interstitial inflammation, fibrosis, hyperplasia of pneumocytes, and foamy macrophages. Respiratory symptoms and abnormal chest X-ray findings were nearly complete cleared after using steroid and withdrawal of amiodarone. We report a case of amiodarone-induced pulmonary toxicity with literature review.

• The Korean Journal of Cytopathology
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• v.17 no.1
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• pp.63-68
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• 2006

Inflammatory myofibroblastic tumor (IMT), normally referred to as inflammatory pseudotumor, is a fairly rare condition. Fine needle aspiration cytology (FNAC) of IMT has only rarely been reported. Here, we describe one such case of pulmonary inflammatory myofibroblastic tumor. A 30-year-old man presented with a 2.8cm-sized mass in his lung. Chest CT revealed a well defined, poorly enhancing mass. FNAC showed some fascicular or swirled clusters of spindle cells, admixed with occasional inflammatory cells and foamy histiocytes. The majority of the tumor cells evidenced bland, elongated nuclei, but infrequent pleomorphic nuclei. Some of the tumor cells evidenced nuclear grooves and intranuclear inclusions. Although the cytological differentiation of IMT from malignant lesions is not immensely problematic, due to the general paucity of cytological and nuclear atypia, a definite cytological diagnosis of IMT cannot be rendered simply by FNAC. Therefore, a diagnosis of IMT may be suggested via exclusive diagnosis.

• Journal of Radiation Protection and Research
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• v.42 no.4
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• pp.177-188
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• 2017

Background: Radiation-induced pneumonitis and pulmonary fibrosis are common dose-limiting complications in patients receiving radiotherapy for lung, breast, and lymphoid cancers. In this study, we investigated the characteristics of effective immune cells related to pneumonitis and fibrosis after irradiation. Materials and Methods: After anesthesia, the whole thorax of C57BL/6 mice was irradiated at 14 Gy. The lung tissue and bronchoalveolar lavage fluid were collected at defined time points post-irradiation for the determination of histological and immunohistochemical analysis and inflammatory cell population infiltrated into the lung. Results and Discussion: Whole thoracic irradiation increased the deposition of extracellular matrix (ECM), lung weight, and pleural effusions, which started to die from 4 months later. At 4 months after irradiation, the numbers of macrophages and lymphocytes as well as neutrophils were increased dramatically in the lung. Interestingly, the macrophages that were recruited into the lung after irradiation had an enlarged foamy morphology. In addition, the expressions of chemokines (CCL-2, CCL-3, CXCL-10) for the attraction of macrophages and T cells were higher in the lung of irradiated mice. The high expressions of these chemokines were sustained up to 6 months following irradiation. In thoracic irradiated mice, infiltrated macrophages into the lung had the high levels of Mac-3 antigens on their surface and upregulated the hallmarks of alternatively activated macrophages such as arginase-1 and CD206. Furthermore, the levels of IL-4 and IL-13 were higher in a BAL fluid of irradiated mice. Conclusion: All results show that thoracic irradiation induces to infiltrate various inflammation-related immune cells, especially alternatively activated macrophages, through enhancing the expression of chemokines, suggesting that alternatively activated macrophages are most likely important for leading to pulmonary fibrosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.4
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• pp.263-267
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• 2017

Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts. A dried blood spot test revealed markedly decreased activity of LAL. LIPA gene sequencing identified the presence of a novel homozygous mutation (p.Thr177Ile). The patient's elevated liver enzymes and dyslipidemia improved with enzyme replacement therapy. This is the first report of a Korean child with LAL deficiency, and our findings suggest that this condition should be considered in the differential diagnosis of children with hepatosplenomegaly and dyslipidemia.

• Journal of fish pathology
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• v.24 no.3
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• pp.225-236
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• 2011

This study was aimed to determine the fish physical status according to the gross and histopathological findings of liver in cultured black rock fish, Sebastes schlegeli. All 47 fish submitted had no marked abnormalities in the external findings. 42.55% of fish showed normal liver, 25.53% yellow liver, 25.53% atrophic brown liver, 4.26% yellowish-green liver and 2.13% fatty liver in gross examination. Grossly normal liver showed no remarkable change in lobular structure but many vacuoles were found in hepatic cell. Hepatic cells took normal roundish, polygonal shapes containing spherical nuclei. In group of yellow-brown liver, many brown pigments were seen in hepatic cells, MMCs and brown-colored hyaline droplets within cytoplasm of hepatocytes. Yellowish green pigments were seen in hepatic cells and MMCs of yellow green liver and green colored hyaline droplets within hepatocytes. The dilated central veins are highlighted with atrophy of hepatic cells. Outline of atrophic hepatocyte became ambiguous and nucleus frequently become small and pyknotic. Fatty liver showed prominent vacuolar structures in cells as clear spaces or foamy cytoplasm with degenerative nuclei. From these results, it was strongly suggested that hepatic gross and histological findings could be used as important and critical health parameters of fish prior to progression to substantial manifestation as clinical disease.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.19 no.8
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• pp.137-145
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• 2018

In this study, we categorized the subjective recognition of 20's young generation about virtual currency into a few meaningful types and analyzed the characteristics of each type using the Qmethodology. For the Qpopulation, we selected a total of 337 samples which were derived from the Internet search, in-depth interviews, and literature survey, chose the final 51 Qstatements, and performed the Q-classification for the 40 P samples of 20's, including university students. Then, we analyzed the results using the PC-QUANL program. From the analysis, we found meaningful differences between each recognition type, and, after classifying the recognition types into four, we named each of them as 'investment-vehicle' type, 'future-technology' type, 'kind of gambling-like plays' type, and 'foamy faddishness' type. We hope that this research result can contribute to follow-up research and social discussion as base materials.

• Clinical and Experimental Pediatrics
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• v.55 no.11
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• pp.438-444
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• 2012

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at $37^{+1}$ weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of ${\beta}$-D-hexosaminidase and ${\alpha}$-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

• Journal of Yeungnam Medical Science
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• v.15 no.2
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• pp.359-370
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• 1998

Eight cases of chonproblastoma were studied by analyzing the clinical and pathologic findings. The age of eight cases ranged from 17 to 38 years old(median age, 22.7 years old). The tumors developed in the femur(3 cases), patella(2 cases), tibia(1 case), fibula(l case), and ulna(1 case). The mean diameter of tumors was 4.0cm (range, 1.5 to 8.0cm). Grossly, tumors showed grayish brown solid area with foci of secondary aneurysmal bone cyst. Histologically, the tumor cells were round or polygonal in shape with nuclear groove. And there were chondroid differentiation(7 cases), mitosis(3 cases), calcific deposits(3 cases), secondary aneurysmal bone cyst(4 cases), hemosiderin deposits(4 cases), necrosis(3 cases), vascular invasion(1 caes), and foamy histiocytes and cholesterol cleft(l cases). All cases showed no metastasis to lymph node and distant organ. Seven cases (87.5%) were immunoreactive for S-100 protein. None was immunoreactive for cytokeratin.